1-MINUTE CONSULT

BRIEF ANSWERS
TO SPECIFIC
CLINICAL

Q: When does pericarditis merit a workup QUESTIONS

for autoimmune or inflammatory disease?

M. CHADI ALRAIES, MD, FACP less common causes include bacterial infec-
Department of Medicine, Division of Cardiology, University of Minnesota,
Minneapolis, MN tion, postpericardiotomy syndrome, myocar-
dial infarction, primary or metastatic tumors,
MOTAZ BAIBARS, MD
Department of Medicine, Peninsula Regional Medical Center, Salisbury, MD trauma, radiation, and uremia. However, peri-
carditis can also be part of the presentation of
ALLAN L. KLEIN, MD
Center for Pericardial Disease, Heart and Vascular Institute, Cleveland Clinic systemic inflammatory and autoimmune dis-
eases such as rheumatoid arthritis and system-

A: Pericarditis is in most cases a one-

time disease simply treated with anti-
inflammatory drugs. It requires no extensive
workup for systemic inflammatory or autoim-
mune disease. Further evaluation is required
for patients who have recurrent pericarditis
resistant to conventional therapy or pericar-
ditis with manifestations of systemic disease.
■■ ACUTE PERICARDITIS
Pericardial disease has different presenta-
tions: acute, recurrent, constrictive, effusive-
constrictive, and pericardial effusion with or
without tamponade. Acute pericarditis is the
most common of these and can affect people
of all ages. The typical acute manifestations
are chest pain (usually pleuritic), a pericardial
friction rub, and widespread ST-segment el-
evation on the electrocardiogram.1,2 The chest
pain tends to be sharp and long-lasting; it radi-
ates to the trapezius ridge and increases during
respiration or body movements.
Acute pericarditis usually responds to an
anti-inflammatory drug such as colchicine
0.6 mg/day for 3 months, a nonsteroidal anti-
inflammatory drug such as ibuprofen 600 mg
three times a day for 10 days, and in advanced
resistant cases, an oral corticosteroid.3,4
Most often, pericarditis is either idiopathic
or occurs after a respiratory viral illness. Much
doi:10.3949/ccjm.82a.14064
C L E V E L A N D C L I N I C J O U R N A L O F M E D I C I N E   V O L U M E 8 2 • N U M B E R 5   M AY 2 0 1 5
Downloaded from www.ccjm.org on January 8, 2022. For personal use only. All other uses require permission.
ic lupus erythematosus; hereditary periodic fe-
ver syndromes such as familial Mediterranean
fever; and systemic-onset juvenile idiopathic
arthritis.1,5
In acute pericarditis, a complex workup is
usually not justified, since the results will have
limited usefulness in the clinical management
of the patient. It is most often diagnosed by
Patients with
the presenting symptoms, auscultation, elec- recurrent
trocardiography, echocardiography, and chest pericarditis and
radiography, and by additional basic tests that
include a complete blood cell count, complete pericarditis
metabolic profile, erythrocyte sedimentation with manifesta-
rate, and C-reactive protein level. However,
if pericarditis does not respond to anti-inflam-
tions of system-
matory treatment and if an autoimmune or ic disease need
infectious disease is suspected, further evalu- a thorough
ation may include antinuclear antibody test-
ing and testing for human immunodeficiency workup for
virus and tuberculosis. If the diagnosis of acute autoimmune
pericarditis remains uncertain, cardiac mag-
netic resonance imaging (MRI) may be useful.
disease
■■ RECURRENT PERICARDITIS
Although acute pericarditis most often has
a benign course and responds well to anti-
inflammatory drugs, 20% to 30% of patients
who have a first attack of acute pericarditis
have a recurrence, and up to 50% of patients
who have one recurrence will have another.3,4
Disease activity can be followed with se-
285
 PERICARDITIS WORKUP
rial testing of inflammatory markers—eg,
erythrocyte sedimentation rate and C-reac-
tive protein level. Echocardiography, cardiac
computed tomography, and cardiac MRI can
characterize active inflammation, edema, peri-
cardial thickness, and pericardial effusion.6–8
Recurrent pericarditis is often resistant
to standard therapy and requires corticoste-
roids in high doses, which paradoxically can
increase the risk of recurrence. Therefore,
further workup for underlying autoimmune
disease, systemic inflammatory disease, or in-
fection is necessary. More potent immunosup-
pressive therapy may be required, not only in
pericarditis associated with systemic autoim-
mune or inflammatory conditions, but even
in idiopathic recurrent pericarditis, either to
control symptoms or to mitigate the effects of
corticosteroids.
■■ SYSTEMIC INFLAMMATION
The true prevalence of pericardial disease in
most systemic inflammatory and autoimmune
diseases is difficult to determine from current
data. But advances in serologic testing and im-
aging techniques have shown cardiac involve-
ment in a number of inflammatory diseases.9
In one study, a serologic autoimmune work-
up in patients with acute pericarditis found that
2% had collagen vascular disease.9 Pericardial
involvement is likely in systemic lupus erythe-
matosus,10 and a postmortem study of patients
with systemic sclerosis found that 72% had
pericarditis.11 Mixed connective tissue disease
has been associated with pericarditis in 29% of
cases and 56% in autopsy studies.12,13 Pericar-
■■ REFERENCES
1. Troughton RW, Asher CR, Klein AL. Pericarditis. Lancet 2004; 363:717–727.
2. Alraies MC, Klein AL. Should we still use electrocardiography to diag-
nose pericardial disease? Cleve Clin J Med 2013; 80:97–100.
3. Imazio M, Brucato A, Cemin R, et al; ICAP Investigators. A randomized
trial of colchicine for acute pericarditis. N Engl J Med 2013; 369:1522–
1528.
4. Imazio M, Cecchi E, Demichelis B, et al. Indicators of poor prognosis of
acute pericarditis. Circulation 2007; 115:2739–2744.
5. Zayas R, Anguita M, Torres F, et al. Incidence of specific etiology and
role of methods for specific etiologic diagnosis of primary acute pericar-
ditis. Am J Cardiol 1995; 75:378–382.
6. Verhaert D, Gabriel RS, Johnston D, Lytle BW, Desai MY, Klein AL.
The role of multimodality imaging in the management of pericardial
disease. Circ Cardiovasc Imaging 2010; 3:333–343.
7. Klein AL, Abbara S, Agler DA, et al. American Society of Echocardiogra-
phy clinical recommendations for multimodality cardiovascular imaging
of patients with pericardial disease: endorsed by the Society for Cardio-
vascular Magnetic Resonance and Society of Cardiovascular Computed
286 C L E V E L A N D C L I N I C J O U R N A L O F M E D I C I N E   V O L U M E 8 2 • N U M B E R 5   M AY 2 0 1 5
Downloaded from www.ccjm.org on January 8, 2022. For personal use only. All other uses require permission.
ditis may be the initial manifestation of vascu-
litis—eg, Takayasu arteritis or granulomatosis
with polyangiitis (formerly known as Wegener
granulomatosis).
Other diseases with pericardial involve-
ment include Still disease, Sjögren syndrome,
sarcoidosis, and inflammatory bowel disease.
Symptomatic pericarditis occurs in about 25%
of patients with Sjögren syndrome and asymp-
tomatic pericardial involvement in more than
half. Autopsy studies reported pericardial in-
volvement in up to 80% of patients with sys-
temic lupus erythematosus. Cardiac tampon-
ade occurs in fewer than 2%, and constrictive
pericarditis is extremely rare.5,9–11
■■ RECOMMENDATIONS
Patients with a first episode of pericarditis
should be treated with an anti-inflammatory
medication, with no comprehensive testing
for autoimmune disease. An evaluation for
autoimmune and infectious disease should be
carried out in patients with fever (temperature
> 38°C; 100.4°F), recurrent pericarditis, recur-
rent large pericardial effusion or tamponade,
or night sweats despite conventional medical
therapy. Signs of systemic disease such as re-
nal failure, elevated liver enzymes, or skin rash
merit further evaluation.
Prospective studies using appropriate se-
rologic testing and imaging are needed to de-
termine the correlation between myopericar-
dial involvement and inflammatory diseases
because of increased morbidity and mortality
in several of these diseases. ■
Tomography. J Am Soc Echocardiogr 2013; 26:965–1012.e15.
8. Yingchoncharoen T, Alraies MC, Kwon DH, Rodriguez ER, Tan CD,
Klein AL. Emerging role of multimodality imaging in management of
inflammatory pericardial diseases. Expert Rev Cardiovasc Ther 2013;
11:1211–1225.
9. Knockaert DC. Cardiac involvement in systemic inflammatory diseases.
Eur Heart J 2007; 28:1797–1804.
10. Doria A, Iaccarino L, Sarzi-Puttini P, Atzeni F, Turriel M, Petri M. Cardiac
involvement in systemic lupus erythematosus. Lupus 2005; 14:683–686.
11. Byers RJ, Marshall DA, Freemont AJ. Pericardial involvement in systemic
sclerosis. Ann Rheum Dis 1997; 56:393–394.
12. Kasukawa R. Mixed connective tissue disease. Intern Med 1999;
38:386–393.
13. Bezerra MC, Saraiva F Jr, Carvalho JF, Caleiro MT, Goncalves CR, Borba EF.
Cardiac tamponade due to massive pericardial effusion in mixed connec-
tive tissue disease: reversal with steroid therapy. Lupus 2004; 13:618–620.
ADDRESS: M. Chadi Alraies, MD, FACP, Department of Medicine, Division
of Cardiology, University of Minnesota, 420 Delaware Street SE, MMC 506,
Minneapolis, MN 55455; e-mail: alraies@hotmail.com